(M) Week 9: Test for red cell energy and metabolic defect

Question 1

A screening test used to determine the presence of normal activity of the G-6-PD and Pyruvate kinase in the blood

Answer 1

Florescent spot test

Question 2

this deficiency causes acute hemolytic anemia after the patient is exposed to an oxidizing substance produced from the metabolized administered drugs

Answer 2

G-6-PD

Question 3

drugs such as certain antibiotics and anti-malarial drugs form peroxide which oxidizes the _______________ in the absence of G-6PD enzyme forming _______________-

Answer 3

hemoglobin
heinz bodies

Question 4

match the principle to the test

approximately 1 ml drop of anticoagulated whole blood using either EDTA, Heparin or ACD anticoagulant, is placed to a special filter paper which is then mixed with a drop of a test reagent containing glucose-6-phosphate and NADP

a. Florescent spot test
b. glutathione reduction test
c. acute hemolysis test
d. ascorbate cyanide test

Answer 4

a

Question 5

if there’s enough level of G-6-PD a chemical reaction will occur which converts the NADP to

Answer 5

reduced NADP or NADPH

Question 6

there’s fluorescence if NADPH is absent/present

Answer 6

present

Question 7

major enzyme in the EMP pathway, responsible for the production of enough ATP

Answer 7

Pyruvate kinase

Question 8

Deficiency of this enzyme may cause the instability of the red cell
membrane due to the insufficient production of energy that eventually leads to hemolytic anemia.

Answer 8

pyruvate kinase

Question 9

match the principle to the test:

A drop of blood is placed in a filter paper and mixed with a test reagent
containing phosphoenol pyruvate, ADP, and reduced nicotinamide-adenine dinucleotide or NAD

a. Florescent spot test
b. glutathione reduction test
c. acute hemolysis test
d. ascorbate cyanide test

Answer 9

a

Question 10

If there is enough pyruvate kinase in the blood a chemical reaction will occur that leads to the conversion
of NADH to___________

Answer 10

NAD

Question 11

the presence of NAD (hinders/promotes) fluorescence

Answer 11

hinders

Question 12

what is the positive result for pyruvate kinase enzyme

Answer 12

no fluorescence

Question 13

What are the factors that may cause a false normal for pyruvate kinase and G-6PD tests?

Answer 13

high WBC (>20,000/ul)
high platelet count

Question 14

what are the factors that may cause a false normal result for G-6PD testing

Answer 14

high levels of reticulocyte

Question 15

what may cause for pyruvate kinase test to have a false positive result

Answer 15

plasma

Question 16

what effect does hemoglobin have on fluorescence

Answer 16

quenching

Question 17

if the hematocrit of the person is more than 50% it is recommended that _________ of the required amount of specimen is used

Answer 17

only half

Question 18

if the hematocrit is__________ the amount of specimen should be doubled.

Answer 18

less than 20%

Question 19

which test has the same principle as the fluorescent spot test

Answer 19

glutathione reduction test

Question 20

If the blood is containing normal levels of Glutathione reductase (GSSG-R) the NADPH will be converted to

Answer 20

NADP

Question 21

if there’s a normal level of glutathione in the blood, the sample will fluoresce for ___________–

Answer 21

20 minutes

Question 22

in glutathione reductase deficiency the fluorescence will persist and continue for _____________

Answer 22

more than one hour

Question 23

This test is used to screen the two most common red cell enzymopathy and these are the G-6-
PD deficiency and pyruvate kinase deficiency.

Answer 23

auto hemolysis test

Question 24

G6PD/PK DEFICIENCY

what is the test for this principle?

Blood specimen is subjected into warm temperature
ranging from 37OC to 40OC for 48 hours

Answer 24

autohemolysis test

Question 25

autohemolysis test

if the blood is deficient with either of the G6PD or pyruvate kinase, the red cells will be

Answer 25

lysed => reddish plasma

Question 26

what is used to correct the occurences of hemolysis in autohemolysis test

Answer 26

glucose reagent or ATP reagent

Question 27

If the hemolysis is corrected by adding glucose reagent but not with ATP
reagent, then the patient has

Answer 27

G-6-PD deficiency ( Auto hemolysis type I,)

Question 28

if the hemolysis is not corrected by the glucose reagent but corrected by the ATP reagent, then the patient has

Answer 28

pyruvate kinase deficiency (s Auto hemolysis type II,)

Question 29

If both the glucose and ATP reagent corrected the hemolysis, then the patient is suffering from

Answer 29

hereditary spherocytosis (s Auto hemolysis type III)

Question 30

Test used to diagnose an autoimmune disorder known as Paroxysmal
Cold hemoglobinuria (PCH)

Answer 30

Donath-Landsteiner test

Question 31

a condition where a person produces an anti-P autoantibody

Answer 31

PCH

Question 32

infections that trigger the production of anti-P autoantibody

Answer 32

syphilis

Question 33

auto anti-P usually binds with red cell membrane at ____________ and triggers red cell hemolysis at ____________ by activating the complement system

Answer 33

cold temp
37 deg c

Question 34

Which test has this principle?

uses two separate tubes filled with whole blood. One of the tubes is
incubated at 37OC for 60 minutes which is the control tube and the other tube is incubated first at 0-4
OC for 30 minutes then later at 37OC for 60 minutes

Answer 34

Donath-Landsteiner test

Question 35

positive result for Donald-Landsteiner test

Answer 35

incubated - hemolysis (at 37 degree)
no hemolysis on control tube

Question 36

negative result for Donald-Landsteiner test

Answer 36

no hemolysis on both tubes

Question 37

What is the interpretation if both tubes showed bemolysis in the Donald-Landsteiner test

Answer 37

negative for PCH

Question 38

test is used to determine the in vivo attachment of an
auto or allo antibody and complement fragments on red cell membrane

Answer 38

Direct Antihuman Globulin Test (DAT)

Question 39

in vivo attachment of an auto or allo antibody and complement fragments on red cell membrane occurs on the following conditions:

Answer 39

autoimmune hemolytic anemia
hemolytic transfusion reactions
hemolytic disease of the newborn
PCH

Question 40

What reagent is used by the direct antihuman globulin test

Answer 40

Antihuman globulin (AHG) or Coomb’s reagent